ALSFRS-R is 12-item scale used to assess disability and to measure disease progression in ALS patients. The objective is to validate the Arabic version of ALSFRS-R based on the original English version. This is a cross sectional study. ALSFRS-R was administered to 162 Egyptian patients with ALS after being translated in Arabic, and reapplied after 1 week. Patients were recruited from 2 centers: Neuromuscular unit, Ain Shams University hospitals and the specialized ALS clinic which is located at the international medical center (IMC). No significant differences were found between the application and reapplication of the scale ( = 0.5). The linear regression and internal consistency that were measured by Pearson correlation and alpha Conbrach respectively were significant. The Arabic version of the ALSFRS-R proposed by the current was proven to be reproducible and valid among Egyptian ALS patients. Thus, it will provide a useful tool for professionals to evaluate Arabic speaking patients in clinical practice and research.
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http://dx.doi.org/10.1080/21678421.2020.1815788 | DOI Listing |
Muscle Nerve
January 2025
Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Introduction/aims: Spirometry is the conventional means to measure lung function in amyotrophic lateral sclerosis (ALS), but is dependent on patient effort and bulbar strength. We aimed to use electric impedance tomography (EIT), an emerging non-invasive imaging modality, to measure dynamic lung volume changes.
Methods: Twenty-one patients with ALS underwent sitting and supine spirometry for forced vital capacity (FVC), and sitting and supine EIT.
J Neuroinflammation
January 2025
Memory Unit, Neurology Department and Institut de Recerca Sant Pau, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Sant Quintí 77-79, 08041, Barcelona, Spain.
Background: Neuroinflammation plays a major role in amyotrophic lateral sclerosis (ALS), and cumulative evidence suggests that systemic inflammation and the infiltration of immune cells into the brain contribute to this process. However, no study has investigated the role of peripheral blood immune cells in ALS pathophysiology using single-cell RNA sequencing (scRNAseq).
Methods: We aimed to characterize immune cells from blood and identify ALS-related immune alterations at single-cell resolution.
Front Immunol
January 2025
Department of Neurosciences, Ospedale Civile di Baggiovara, Azienda Ospedaliero-Universitaria di Modena, Modena, Italy.
Introduction: T regulatory cells (Tregs) inversely correlate with disease progression in Amyotrophic Lateral Sclerosis (ALS) and fast-progressing ALS patients have been reported to exhibit dysfunctional, as well as reduced, levels of Tregs. This study aimed to evaluate the longitudinal changes in Tregs among ALS patients, considering potential clinical and biological modifiers of their percentages and concentrations. Additionally, we explored whether measures of ALS progression, such as the decline over time in the revised ALS Functional Rating Scale (ALSFRS-r) or forced vital capacity (FVC) correlated Treg levels and whether Treg phenotype varied during the course of ALS.
View Article and Find Full Text PDFTher Adv Neurol Disord
January 2025
Department of Neurology, Ulm University, Ulm, Germany.
The advent of antisense oligonucleotide (ASO) therapies in neurodegenerative disorders is associated with enormous hope. Nusinersen treatment was a breakthrough intervention in the recessive disease spinal muscular atrophy, and superoxide dismutase 1 (SOD1) amyotrophic lateral sclerosis (ALS) seems to be the paradigm disease in dominant degenerative diseases. The results of treatment with the ASO tofersen in SOD1-ALS show that the drug has a convincing beneficial effect on ALS caused by SOD1 mutations, that preclinical studies in rodents predicted the therapeutic effect in the human disease, and that clinical efficacy is associated with a specific sequence of effects of the drug on mechanistic and degenerative biomarkers and, subsequently, functional outcomes such as weight stabilization and ALSFRS-R.
View Article and Find Full Text PDFPLoS One
January 2025
CNRS, CRMBM, Aix Marseille Univ, Marseille, France.
Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by significant heterogeneity among patients. 23Na MRI maps abnormal sodium homeostasis that reflects metabolic alterations and energetic failure contributing to the neurodegenerative process. In this study, we investigated disease severity at the individual level in ALS patients using brain 23Na MRI.
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